scholarly journals EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR)

2009 ◽  
Vol 68 (5) ◽  
pp. 620-628 ◽  
Author(s):  
O Kowal-Bielecka ◽  
R Landewé ◽  
J Avouac ◽  
S Chwiesko ◽  
I Miniati ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Clinical Research ◽  
Research Group ◽  
Task Force ◽  
Multiple Organ ◽  
Future Research ◽  
Scleroderma Renal Crisis ◽  
Evidence Based ◽  
Future Research Agenda ◽  
Organ Systems

Purpose:The optimal treatment of systemic sclerosis (SSc) is a challenge because the pathogenesis of SSc is unclear and it is an uncommon and clinically heterogeneous disease affecting multiple organ systems. The aim of the European League Against Rheumatism (EULAR) Scleroderma Trials and Research group (EUSTAR) was to develop evidence-based, consensus-derived recommendations for the treatment of SSc.Methods:To obtain and maintain a high level of intrinsic quality and comparability of this approach, EULAR standard operating procedures were followed. The task force comprised 18 SSc experts from Europe, the USA and Japan, two SSc patients and three fellows for literature research. The preliminary set of research questions concerning SSc treatment was provided by 74 EUSTAR centres.Results:Based on discussion of the clinical research evidence from published literature, and combining this with current expert opinion and clinical experience, 14 recommendations for the treatment of SSc were formulated. The final set includes the following recommendations: three on SSc-related digital vasculopathy (Raynaud’s phenomenon and ulcers); four on SSc-related pulmonary arterial hypertension; three on SSc-related gastrointestinal involvement; two on scleroderma renal crisis; one on SSc-related interstitial lung disease and one on skin involvement. Experts also formulated several questions for a future research agenda.Conclusions:Evidence-based, consensus-derived recommendations are useful for rheumatologists to help guide treatment for patients with SSc. These recommendations may also help to define directions for future clinical research in SSc.

scholarly journals Hydrogen Sulfide: A Therapeutic Option in Systemic Sclerosis

2018 ◽  
Vol 19 (12) ◽  
pp. 4121 ◽  
Author(s):  
Amaal Abdulle ◽  
Harry van Goor ◽  
Douwe Mulder
Keyword(s):  
Hydrogen Sulfide ◽  
Systemic Sclerosis ◽  
Animal Studies ◽  
Therapeutic Option ◽  
Multiple Organ ◽  
Current Data ◽  
Organ Systems ◽  
Transsulfuration Pathway ◽  
Exact Etiology ◽  
Vascular Physiology

Systemic sclerosis (SSc) is a lethal disease that is characterized by auto-immunity, vascular injury, and progressive fibrosis of multiple organ systems. Despite the fact that the exact etiology of SSc remains unknown, oxidative stress has been associated with a large range of SSc-related complications. In addition to the well-known detrimental properties of reactive oxygen species (ROS), gasotransmitters (e.g., nitric oxide (NO), carbon monoxide (CO), and hydrogen sulfide (H2S)) are also thought to play an important role in SSc. Accordingly, the diverse physiologic actions of NO and CO and their role in SSc have been previously studied. Recently, multiple studies have also shown the importance of the third gasotransmitter H2S in both vascular physiology and pathophysiology. Interestingly, homocysteine (which is converted into H2S through the transsulfuration pathway) is often found to be elevated in SSc patients; suggesting defects in the transsulfuration pathway. Hydrogen sulfide, which is known to have several effects, including a strong antioxidant and vasodilator effect, could potentially play a prominent role in the initiation and progression of vasculopathy. A better understanding of the actions of gasotransmitters, like H2S, in the development of SSc-related vasculopathy, could help to create early interventions to attenuate the disease course. This paper will review the role of H2S in vascular (patho-)physiology and potential disturbances in SSc. Moreover, current data from experimental animal studies will be reviewed. Lastly, we will evaluate potential interventional strategies.

scholarly journals Update of EULAR recommendations for the treatment of systemic sclerosis

2016 ◽  
Vol 76 (8) ◽  
pp. 1327-1339 ◽  
Author(s):  
Otylia Kowal-Bielecka ◽  
Jaap Fransen ◽  
Jerome Avouac ◽  
Mike Becker ◽  
Agnieszka Kulak ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Task Force ◽  
Previous Treatment ◽  
Haematopoietic Stem Cell ◽  
Scleroderma Renal Crisis ◽  
Digital Ulcers ◽  
Eular Recommendations ◽  
Delphi Approach ◽  
The Usa ◽  
Clinical Questions

The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis and gastrointestinal involvement. Compared with the 2009 recommendations, the 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc.

scholarly journals Criteria for Defining Polycystic Ovary Syndrome as a Predominantly Hyperandrogenic Syndrome: An Androgen Excess Society Guideline

2006 ◽  
Vol 91 (11) ◽  
pp. 4237-4245 ◽  
Author(s):  
Ricardo Azziz ◽  
Enrico Carmina ◽  
Didier Dewailly ◽  
Evanthia Diamanti-Kandarakis ◽  
Hector F. Escobar-Morreale ◽  
...  
Keyword(s):  
Polycystic Ovary Syndrome ◽  
Task Force ◽  
Polycystic Ovary ◽  
Future Research ◽  
Evidence Based ◽  
Consensus Process ◽  
Androgen Excess ◽  
Ovary Syndrome ◽  
Initial Report ◽  
New Research

Abstract Objective: The Androgen Excess Society (AES) charged a task force to review all available data and recommend an evidence-based definition for polycystic ovary syndrome (PCOS), whether already in use or not, to guide clinical diagnosis and future research. Participants: Participants included expert investigators in the field. Evidence: Based on a systematic review of the published peer-reviewed medical literature, by querying MEDLINE databases, we tried to identify studies evaluating the epidemiology or phenotypic aspects of PCOS. Consensus Process: The task force drafted the initial report, following a consensus process via electronic communication, which was then reviewed and critiqued by the AES Board of Directors. No section was finalized until all members were satisfied with the contents and minority opinions noted. Statements that were not supported by peer-reviewed evidence were not included. Conclusions: Based on the available data, it is the view of the AES Task Force on the Phenotype of PCOS that there should be acceptance of the original 1990 National Institutes of Health criteria with some modifications, taking into consideration the concerns expressed in the proceedings of the 2003 Rotterdam conference. A principal conclusion was that PCOS should be first considered a disorder of androgen excess or hyperandrogenism, although a minority considered the possibility that there may be forms of PCOS without overt evidence of hyperandrogenism but recognized that more data are required before validating this supposition. Finally, the task force recognized, and fully expects, that the definition of this syndrome will evolve over time to incorporate new research findings.

scholarly journals Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease

Blood ◽  
2014 ◽  
Vol 124 (4) ◽  
pp. 483-492 ◽  
Author(s):  
Eli L. Diamond ◽  
Lorenzo Dagna ◽  
David M. Hyman ◽  
Giulio Cavalli ◽  
Filip Janku ◽  
...  
Keyword(s):  
Clinical Management ◽  
Clinical Laboratory ◽  
Clinical Manifestations ◽  
Multiple Organ ◽  
Future Research ◽  
Consensus Guidelines ◽  
Erdheim Chester Disease ◽  
Organ Systems ◽  
Erdheim Chester ◽  
Chester Disease

Abstract Erdheim-Chester disease (ECD) is a rare, non-Langerhans histiocytosis. Recent findings suggest that ECD is a clonal disorder, marked by recurrent BRAFV600E mutations in >50% of patients, in which chronic uncontrolled inflammation is an important mediator of disease pathogenesis. Although ∼500 to 550 cases have been described in the literature to date, increased physician awareness has driven a dramatic increase in ECD diagnoses over the last decade. ECD frequently involves multiple organ systems and has historically lacked effective therapies. Given the protean clinical manifestations and the lack of a consensus-derived approach for the management of ECD, we provide here the first multidisciplinary consensus guidelines for the clinical management of ECD. These recommendations were outlined at the First International Medical Symposium for ECD, comprised of a comprehensive group of international academicians with expertise in the pathophysiology and therapy of ECD. Detailed recommendations on the initial clinical, laboratory, and radiographic assessment of ECD patients are presented in addition to treatment recommendations based on critical appraisal of the literature and clinical experience. These formalized consensus descriptions will hopefully facilitate ongoing and future research efforts in this disorder.

scholarly journals Vitamin D Supplementation: A Potential Prevention Method against Contagious Viral Respiratory Illnesses

2021 ◽  
Vol 04 (10) ◽  
Author(s):  
Michael Ausi ◽  
Keyword(s):  
Vitamin D ◽  
Immune Cells ◽  
Task Force ◽  
The United States ◽  
Future Research ◽  
Evidence Based ◽  
T Helper ◽  
Respiratory Illnesses ◽  
Adaptive Immune Cells ◽  
Viral Respiratory

This narrative review explores the protective value of Vitamin D (VD) and its metabolites, such as Vitamin D2 (VD2) and Vitamin D3 (VD3), and their preventive health potentials backed by evidence-based medicine against viral respiratory illnesses (VRI). With the recent events of late 2019, a severe acute respiratory illness began to sweep the globe. This SARS illness was of coronavirus-2 variant known as SARS-CoV-2 (COVID-19). With COVID-19 in mind, the purpose of this paper was to explore a preventative health model utilizing evidence-based medical research to safeguard against similar viral catastrophes. The knowledge gained is essential for the future education of the general population and necessary for a more preemptive and protective approach to VRIs. Google Scholar, PubMed, and DuckDuckgo were the most often utilized internet search engines and databases to collect sources. Twelve evidence-based medical articles were selected based on specific inclusionary and exclusionary criteria. More specifically, eleven systematic-review and meta-analyses (SRMA) and one Randomized Controlled Trial (RCT) were selected. The data extracted from the eleven articles were analyzed to understand the relationship between VD, VD3, and Viral Respiratory illnesses (VRIs). VD is known to have various protective actions on the immune system (IS). First, VD activates gene transcription upon innate immune cells to increase viral defensive proteins such as Defensins and Cathelicidins (Laaski, 2011; Liu, 2006; NIH, 2020). More importantly, VD down-regulates certain specific adaptive immune cells named T helper 2 (Th2). The Th2 cellular pathway indirectly downregulates T helper 1 (Th1), cell pro-inflammatory cytokine storms, thus assisting with viral clearance (Huang, Wang, Li et al., 2020). More EBM research displays an inverse relationship between VD status and VRI, like incidences, severity, and duration of VRIs (Abioye, Bromage & Fawz 2021). Recommendations for future research were presented to understand VD better and its possible use as a potential therapeutic or preventive medicine modality for the public. Proposals on health policy changes within health agencies like the United States Preventative Task Force (USPTF) were also provided. Changes included mandatory health VD screens tables and their metabolites for patients of different ages and breeds to achieve proper VRI protection to prevent future pandemics.

scholarly journals Sudden Cardiac Death in Systemic Sclerosis: Diagnostics to Assess Risk and Inform Management

Diagnostics ◽  
2021 ◽  
Vol 11 (10) ◽  
pp. 1781
Author(s):  
Laura Ross ◽  
Elizabeth Paratz ◽  
Murray Baron ◽  
André La Gerche ◽  
Mandana Nikpour
Keyword(s):  
Heart Disease ◽  
Systemic Sclerosis ◽  
Sudden Cardiac Death ◽  
Cardiac Disease ◽  
Cardiac Death ◽  
Diffuse Myocardial Fibrosis ◽  
Future Research ◽  
Disease Course ◽  
Future Research Agenda ◽  
Increased Risk

Cardiac disease is a leading cause of death in systemic sclerosis (SSc) and sudden cardiac death (SCD) is thought to occur more commonly in SSc than in the general population. Diffuse myocardial fibrosis, myocarditis and ischaemic heart disease are all prevalent in SSc and can be reasonably hypothesised to contribute to an increased risk of SCD. Despite this, SCD remains a relatively understudied area of SSc with little understood about SSc-specific risk factors and opportunities for primary prevention. In this review, we present an overview of the possible mechanisms of SCD in SSc and our current understanding of how each of these mechanisms may contribute to cardiac death. This review highlights the need for a future research agenda that addresses the underlying epidemiology of SCD in SSc and identifies opportunities for intervention to modify the disease course of heart disease in SSc.

scholarly journals The Need for Basic, Translational, and Clinical Research in the Field of Hypertrophic Scars

2021 ◽  
Author(s):  
Bonnie C. Carney ◽  
Jeffrey W. Shupp ◽  
Taryn E. Travis
Keyword(s):  
Clinical Research ◽  
Treatment Effectiveness ◽  
Science Research ◽  
Future Research ◽  
Traumatic Injuries ◽  
Matrix Deposition ◽  
Organ Systems ◽  
Extracellular Matrix Deposition

Hypertrophic scar (HTS) is a fibrotic skin disorder that is marked by excessive inflammation and extracellular matrix deposition in response to cutaneous traumatic injuries such as burns, lacerations, incisions, and abrasions. HTS has various risk factors, available treatments, and treatment effectiveness. Research at the basic, translational, and clinical levels are in their infancy compared to fibrotic diseases in other organ systems. This chapter will review current in vitro and in vivo modeling, and highlight research needs to address gaps in the study of HTS. The following topics will be discussed in the chapter: a. Basic Science Research i. Seminal findings ii. Limitations to these models iii. Suggestions for topics of future research b. Translational Science Research i. Seminal findings ii. Limitations to these models iii. Suggestions for topics of future research c. Clinical Research i. Seminal findings ii. Limitations to these models iii. Suggestions for topics of future research.

Critical Care Nursing

2017 ◽  
Keyword(s):  
Critical Care ◽  
Kidney Injury ◽  
Multiple Organ ◽  
Major Incident ◽  
Organ Support ◽  
Evidence Based ◽  
Care Environment ◽  
Organ Systems ◽  
General Aspects ◽  
The Impact

This textbook encompasses the knowledge, skills, and expertise needed to deliver excellent nursing care to critically ill patients. Emphasis is placed on a holistic and compassionate approach towards humanizing the impact of the environment, organ support, and monitoring, as well as critical illness itself. Chapters cover the general aspects of critical care such as the critical care environment or critical care continuum and specific organ systems and diseases. The structure of the systems chapters reminds the reader of the underlying anatomy and physiology as well as highlighting areas of particular relevance to critical care. The focus on priorities for management builds on the ABCDE assessment and offers insight into key interventions in urgent situations as well as outlining evidence-based practice. The book is ideal for those new to the critical care environment, but will also act as a reminder for more experienced nurses when faced with a new situation or when teaching/mentoring students. The patient and their family remain the centre of all This new edition brings the definitions, pathophysiology, and management of fast-changing and challenging areas such as ARDS, sepsis and multiple organ dysfunction, resuscitation, and acute kidney injury up to date as well as including any evidence-based changes associated with nursing practice in critical care. A new chapter covers major incident planning and management and the role of critical care in pandemic situations.

scholarly journals The OMERACT Ultrasound Task Force — Advances and Priorities

2009 ◽  
Vol 36 (8) ◽  
pp. 1829-1832 ◽  
Author(s):  
MARIA-ANTONIETTA D’AGOSTINO ◽  
PHILIP G. CONAGHAN ◽  
ESPERANZA NAREDO ◽  
PHILIPPE AEGERTER ◽  
ANNAMARIA IAGNOCCO ◽  
...  
Keyword(s):  
Scoring System ◽  
Research Agenda ◽  
Task Force ◽  
Interobserver Reliability ◽  
Research Priorities ◽  
Added Value ◽  
Future Research ◽  
Future Research Agenda ◽  
Further Development ◽  
Deep Target

This article reports the most recent work of the OMERACT Ultrasound Task Force (post OMERACT 8) and highlights of future research priorities discussed at the OMERACT 9 meeting, Kananaskis, Canada, May 2008. Results of 3 studies were presented: (1) assessing intermachine reliability; (2) applying the scoring system developed in the hand to other joints most commonly affected in rheumatoid arthritis (RA); and (3) assessing interobserver reliability on a deep target joint (shoulder). Results demonstrated good intermachine reliability between multiple examiners, and good applicability of the scoring system for the hand on other joints (including shoulder). Study conclusions were discussed and a future research agenda was generated, notably the further development of a Global OMERACT Sonography Scoring (GLOSS) system in RA, emphasizing the importance of testing feasibility and added value over standard clinical variables. Future disease areas of importance to develop include a scoring system for enthesitis and osteoarthritis.

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